Phenyl α-L-iduronide cyclohexylammonium salt is used as an enzyme substrate. The activities of alpha-L-iduronidase (EC 188.8.131.52) have been measured using phenyl-α-L-iduronide as the substrate. Measurement of α-L-iduronidase activity using phenyl α-L-iduronide may enable a reliable diagnosis for Hurler syndrome to be made within 15 to 18 days after amniocentesis. Recent studies suggest that 4-Methylumbelliferyl α-L-iduronide (sc-262101) may provide a more sensitive assay for the estimation of α-l-iduronidase in cultured cells and could be used to diagnose Hurler's disease.
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